Fluctuating Vision Loss, Seizures, and Left Parieto-Occipital Mass

2021 ◽  
pp. 22-25
Author(s):  
Alicja Kalinowska-Lyszczarz ◽  
W. Oliver Tobin ◽  
Yong Guo ◽  
Claudia F. Lucchinetti

A 35-year-old man sought care for progressive visual disturbance. Magnetic resonance imaging of the brain showed a large, left-sided, parieto-occipital, contrast-enhancing lesion. He was treated with dexamethasone with brief improvement in vision. Within 5 days he had progressive vision worsening. Two weeks after the onset of his symptoms, brain magnetic resonance imaging showed a decrease in lesion size, and corticosteroids were discontinued. Two months after symptom onset he was found to have alexia without agraphia, and follow-up magnetic resonance imaging showed an increased size of the lesion. Two months after disease onset, the patient underwent a left occipital brain biopsy, which demonstrated a macrophage-enriched active demyelinating lesion with relative axonal sparing. Right arm weakness and aphasia developed, along with a fever. He was treated with dexamethasone. Electroencephalography indicated multiple seizures. Repeated cerebrospinal fluid analysis showed a slightly increased white blood cell count, increased protein level, immunoglobulin G index of 0.84, and the presence of 3 cerebrospinal fluid-unique oligoclonal bands. He was treated with 5 days of intravenous methylprednisolone and levetiracetam, with improvement. Three and a half years later, the patient came to the emergency department with weakness of the left leg associated with reduced sensation. Spinal magnetic resonance imaging showed a new demyelinating contrast-enhancing lesion from T2 to T7. He was treated with 5 days of intravenous methylprednisolone followed by 6 sessions of plasma exchange, with improvement. A diagnosis of relapsing tumefactive demyelination was made. The patient was subsequently treated with ocrelizumab. Tumefactive demyelinating lesions pose a diagnostic challenge, especially if they are the first manifestations of demyelinating disease. Typically, tumefactive demyelinating lesions are large (>2 cm) and are associated with edema, mass effect, and variable patterns of contrast enhancement.

2021 ◽  
pp. 213-215
Author(s):  
W. Oliver Tobin

A 40-year-old man sought care for a 3-year history of tingling in his cheeks and face, with progressive urinary urgency. He had a 1-year history of progressive binocular diplopia and ataxia. On examination, he had decreased vibration sensation in his toes and a mild broad-based gait. Magnetic resonance imaging of the brain indicated mild fluid-attenuated inversion recovery abnormality with punctate postgadolinium enhancement, primarily in the pons. Spinal fluid analysis showed 2 white blood cells/µL with 95% lymphocytes, protein value of 32 mg/dL, 0 unique oligoclonal bands, and normal immunoglobulin G index. Pontine biopsy showed a polyclonal lymphocytic infiltrate, with a small polyclonal B-cell infiltrate. The patient was treated with intravenous methylprednisolone. His symptoms markedly improved, and repeated brain magnetic resonance imaging showed resolution of the enhancing lesions. Six months later he had progressive ataxia and pseudobulbar affect. Repeated brain magnetic resonance imaging showed a recurrence of the punctate enhancing lesions in the pons. A diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids was made. The patient was treated with weekly intravenous methylprednisolone, oral methotrexate, and folic acid supplementation. After 6 weeks, the methylprednisolone infusion interval was lengthened. Serial magnetic resonance imaging for 16 years after the original disease presentation showed no recurrence of enhancing brain lesions. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is an inflammatory brainstem disorder of unknown cause. It presents with a progressive pontocerebellar dysfunction associated with punctate enhancing lesions centered on the pons and cerebellum.


2021 ◽  
Vol 14 ◽  
pp. 117954762198967
Author(s):  
Van Trung Hoang ◽  
Cong Thao Trinh ◽  
Hoang Anh Thi Van ◽  
Thanh Tam Thi Nguyen ◽  
Vichit Chansomphou ◽  
...  

Balo’s concentric sclerosis (BCS) is a rare demyelinating disease known as Multiple Sclerosis (MS) lesion type III. It is a disease of the white matter of the brain characterized by a round lesion with variable concentric myelinated and demyelinated layers, appearing as “onion bulb.” We present a case of BCS and discuss the imaging findings and management strategies of this disease. A 26-y-old male developed headache, weakness, and numbness of limbs. Magnetic resonance imaging (MRI) showed concentric lamellar like demyelinating lesions at the subcortical regions. The patient’s neurological symptoms were consistent with the MRI findings.


2014 ◽  
Vol 3 (10) ◽  
pp. 204798161455221
Author(s):  
Melda Bozluolcay ◽  
Ayse D Elmali ◽  
Sukriye F Menku ◽  
Burcu Zeydan ◽  
Gulcin Benbir ◽  
...  

Background Creutzfeld-Jacob disease (CJD) is a rare, progressive disease that has a vast clinical manifestation range. Cranial magnetic resonance imaging (MRI), electroencephalography (EEG), and measurement of 14-3-3 in cerebrospinal fluid (CSF) may offer a pragmatic approach in the diagnosis of CJD as an alternative to histopathological confirmation. Purpose To present the symptoms and signs of the CJD patients in regard to radiological and neurophysiological findings. Material and Methods We collected all cases with the diagnosis of probable CJD admitted to our neurology department between June 2010 and June 2014. The medical records and laboratory data, clinical features, results of MRI (including diffusion weighted images), EEG and CSF evaluations, and other laboratory data to exclude other possible diagnoses were recorded. None of the patients underwent biopsy or autopsy for histological diagnosis. Results Of 20 patients, 11 (55%) were men and nine (45%) were women. The mean age at disease onset was 60.0 ± 9.5 years (age range, 47–80 years). All patients without exception had characteristic abnormalities in DWI and/or FLAIR on admission, about 4 months after the initial symptom. Periodic complexes on EEGs characteristic for CJD were detected only in 10 patients (50%) on admission and in 13 patients (65%) during disease course. Out of 14 patients who underwent CSF examination, 11 (78.5%) were positive for 14-3-3 protein. Conclusion Although the definite diagnosis of CJD is made histopathologically, we aimed to discuss the value of magnetic resonance imaging in the diagnosis of CJD in respect to EEG findings and protein 14-3-3 levels in CSF.


2021 ◽  
pp. 254-256
Author(s):  
Sara Mariotto ◽  
Silvia Bozzetti ◽  
Maria Elena De Rui ◽  
Fulvia Mazzaferri ◽  
Andrew McKeon ◽  
...  

In March 2020, a 68-year-old man with a history of pulmonary thromboembolism sought care at the emergency department for fever, cough, headache, and confusion. Because of severe respiratory failure, orotracheal intubation was required, and the patient was admitted to the intensive care unit, where bilateral deep vein thrombosis and hematemesis occurred. After 2 weeks, owing to respiratory improvement, the patient was weaned from ventilator support and sedation. However, persistent fluctuations in confusion, anxiety, agitation, and cognitive-motor slowing were noted. One week later, he was referred to the infectious diseases unit, where altered mental status persisted in the absence of fever, seizures, or episodes of impaired consciousness. Chest radiography showed small, bilateral, ground-glass opacities. Brain magnetic resonance imaging showed bilateral involvement of mesial temporal lobes and hippocampus on fluid-attenuated inversion recovery sequences, in the absence of contrast enhancement or restricted diffusion. Nasopharyngeal samples were positive for SARS-CoV-2 on reverse transcriptase–polymerase chain reaction testing. Cerebrospinal fluid examination showed a slight increase in protein concentration, 1 white blood cell/µL, and no evidence of central nervous system infection. In particular, SARS-CoV-2 RNA was not detected. The patient was diagnosed with postinfectious inflammatory (limbic) encephalitis in the course of SARS-CoV-2 infection. The patient was treated with lopinavir/ritonavir and hydroxychloroquine. His recent thromboembolism prevented the administration of intravenous immunoglobulins, and high-dose corticosteroids were not administered because of the recent episode of hematemesis. Improvement in cognitive symptoms was noted 6 weeks after onset. At the time of this writing, May 2020, a few cases of encephalitis after COVID-19 had already been described. These have generally been characterized by fever, cognitive dysfunction, epileptic seizures, coma, and cerebrospinal fluid inflammatory findings. It appears that a common magnetic resonance imaging appearance in these patients is that of diffuse inflammatory encephalitis.


Cephalalgia ◽  
2018 ◽  
Vol 38 (14) ◽  
pp. 1998-2005 ◽  
Author(s):  
Jr-Wei Wu ◽  
Yen-Feng Wang ◽  
Jong-Ling Fuh ◽  
Jiing-Feng Lirng ◽  
Shih-Pin Chen ◽  
...  

Objectives Several brain and spinal magnetic resonance imaging signs have been described in spontaneous intracranial hypotension. Their correlations are not fully studied. This study aimed to explore potential mechanisms underlying cerebral neuroimaging findings and to examine associations among spinal and brain magnetic resonance imaging signs. Methods We conducted a retrospective review of magnetic resonance myelography and brain magnetic resonance imaging records of patients with spontaneous intracranial hypotension. Categorical principal component analysis was employed to cluster brain neuroimaging findings. Spearman correlation was employed to analyze associations among different brain neuroimaging findings and between brain and spinal neuroimaging findings. Results In patients with spontaneous intracranial hypotension (n = 148), categorical principal component analysis of brain neuroimaging signs revealed two clusters: Cerebral venous dilation and brain descent. Among all brain magnetic resonance imaging signs examined, only midbrain-pons angle associated with anterior epidural cerebrospinal fluid collection length (surrogate spinal cerebrospinal fluid leak severity) (n = 148, Spearman’s ρ = −0.38, p < .001). Subgroup analyses showed that the association between midbrain-pons angle (within brain descent cluster) and spinal cerebrospinal fluid leak severity was presented in patients with convex margins of the transverse sinuses (n = 122, Spearman’s ρ = −0.43, p < .001), but not in patients without convex margins (n = 26, Spearman’s ρ = −0.19, p = .348). The association between severity of transverse sinus distension and spinal cerebrospinal fluid leak severity was only presented in patients without convex margins (n = 26, Spearman’s ρ = 0.52, p = .006). Conclusion This study indicates that there are two factors behind the brain neuroimaging findings in spontaneous intracranial hypotension: Cerebral venous dilation and brain descent. Certain brain neuroimaging signs correlate with spinal cerebrospinal fluid leakage severity, depending on different circumstances.


1994 ◽  
Vol 24 (2) ◽  
pp. 525-529 ◽  
Author(s):  
M. H. Hotopf ◽  
S. Pollock ◽  
W. A. Lishman

SynopsisTwo male patients who presented with unusual pictures of dementia in the absence of other obvious symptoms or signs are reported. Investigations demonstrated changes highly suggestive of multiple sclerosis (MS) on magnetic resonance imaging, cerebrospinal fluid analysis and electrophysiological tests. We suggest this represents a rare presentation of multiple sclerosis.


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